Friedreich ataxia, an autosomal recessive condition, ascribed to frataxin gene expansion, has
been shown to result from an iron- induced injury to the mitochondrial respiratory chain.
Buffering free radicals with short-chain quinones (Idebenone) protects the patients against
cardiomyopathy but not CNS involvement. Removing CNS iron should limit the impact of the
neurological symptoms of the disease.